INTRODUCTION: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological disease. There is an incidence. We aimed to evaluate the scientometrics characteristics of the scientific production on BPDCN between 2011 and 2020. METHODOLOGY: A cross-sectional and retrospective bibliometric study was performed. The search was executed in the Scopus database. Data were extracted based on a formula developed using thesaurus MeSH (Medline) and Emtree (Embase) terms. The retrieved papers received 11.2 citations per paper. Four of the ten most productive authors were from the United States. The institution with the highest impact (1064 citations) and the highest scientific output (46 papers) was the University of Texas MD Anderson Cancer Center (United States). RESULTS: The journal “Movement Disorders” rated first with 18 articles and 643 citations in terms of productivity and impact, and articles published in Q1 journals surpassed the remaining quartiles. The most cited articles had national collaboration. Conclusion the number of papers on BPDCN has been rising, yet journals with a higher quality tend to keep the similar publication rates, although they have shown a slight increase in the recent years. CONCLUSIONS: High-income countries' international collaboration is crucial for increasing publications impact; hence, greater collaborations between researchers and institutions from countries around the world are needed to expand knowledge on this subject.

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BACKGROUND: Maternal alloimmunization is associated with adverse outcomes such as hemolytic disease of the fetus and newborn. At-risk pregnant women include those with previous multiple gestations or multiple blood transfusions. This study aimed to determine the proportions and specificities of irregular maternal alloantibodies among antenatal attendees at a federal teaching hospital in Nigeria. An understanding of the pattern of alloimmunization, associated morbidities, and attendant risk factors will guide improved antenatal/perinatal health planning. MATERIALS AND METHODS: A hospital-based, cross-sectional survey was conducted among 150 pregnant women. Data on parity, transfusion history, and other clinical details were obtained with an interviewer administered questionnaire. ABO/Rh D blood groups and hemoglobin phenotypes were retrieved from their antenatal records and confirmed during the study. Alloantibody screening and identification and other serological tests were subsequently performed. Association of independent parameters with other variables was tested using Chi-square analysis or Fisher's exact as appropriate. Level of statistical significance was set at 5% confidence (P = 0.05). RESULTS: Most of the participants (60%) were in their third trimester, while 9.3% were in first trimester of pregnancy. Ninety-one percent of the participants (90.7%) were blood transfusion naïve. Seven of the participants (4.7%) had positive alloantibody screens, of which two (1.33%) were clinically significant maternal alloantibodies (Anti-D and Anti-Lu^b). No statistically significant association was observed between alloimmunization and variables such as gestational age, parity, hemoglobin phenotype, previous blood transfusions, and Rh D negativity. CONCLUSIONS: The authors recommend routine alloantibody screening for at risk pregnancies.

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BACKGROUND: As on March 12, 2020, the WHO declared COVID-19 as a global pandemic. Its rapid spread has posed major challenges to the management of health-care systems. Patients with hematological disorders, being immunocompromised in more ways than one, face a lot of challenges. Most of these patients require frequent visits to health-care facilities for transfusion support, infusions, surveillance, and follow-ups, which increase the risk of exposure and hence infection with severe acute respiratory syndrome coronavirus 2. AIM: We assessed the impact of the pandemic on the decisions of hematologists in Saudi Arabia. Method: An online survey was done through questionnaires, to understand the decisions and course of clinical treatments taken. 45 hematologist answered 20-questions structured questionnaires through online link. RESULTS: The majority of hematologist have used virtual clinics in managing patients and have delayed or canceled well visits. Although some hematologist delayed treatment in stable patients like autologous stem cell transplantation for myeloma patients, the majority did not delay induction or consolidation therapies for patients with leukemia with curative intent plans. CONCLUSION: The crisis brought along with it challenges and opportunities to improve patient care through research and clinical practice. Telemedicine was sought for supporting outpatients. Malignancies were taken care of, with due precautions. Observations of decisions of hematologists resulted in the patients still being closely followed up and urgent treatments being attended to. The hematologists expressed satisfaction with the use of telemedicine. Online consultations and monitoring of patients could probably be taken as an alternative resource in such situations.

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BACKGROUND: Beta (β)-thalassemia is a common single-gene blood disorder resulting in a decrease or absence of β-globin chain synthesis. In β-thalassemia major, an imbalance of α/β globin chains results in severe oxidative stress that leads to pathological conditions. Reactivating the gamma (γ)-globin gene will overcome the excess alpha (α)-globin chains and relieve β-thalassemia patients' clinical course. AIMS AND OBJECTIVES: This study aimed to determine the most competent herbal extract with high efficiency in inducing γ-globin gene expression to facilitate β-thalassemia patients. MATERIALS AND METHODS: This study used K562 cell line culture to assess the fetal hemoglobin (HbF) induction efficacy of Moringa oleifera (MO), Curcuma aueruginosa Roxb. (CA), and Artocarpus altilis (AA). We carried out the benzidine test to count hemoglobin-containing cells and RT-qPCR to measure γ-globin gene expression. RESULTS: The benzidine test showed that MO extract was the highest value (3%) in inducing fetal hemoglobin. However, based on RT-PCR analysis, CA extract had the most significant (2.39 fold change) ability to reactivate the γ-globin gene compared to Hydroxyurea as a positive control. CONCLUSION: The different properties and levels of MO and CA testing in antioxidant and reactivation of gene expression mechanism most probably influenced the discrepancy between the benzidine test and RT-qPCR results.

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INTRODUCTION: Iron-deficiency anemia (IDA) can be grouped under low hepcidin and high erythroferrone (ERFE) anemia. There is a negative correlation between ERFE and hepcidin, irrespective of the type of anemia. ERFE is a mediator of the response to erythropoietic stress, suppressing hepcidin to promote the mobilization of stored iron and the absorption of dietary iron. OBJECTIVE: The objective was to determine the effect of ERFE hormone on hepcidin level as iron metabolism regulator in patients with iron deficiency (ID). METHODS: The study included 50 female patients with ID who were investigated for complete blood count, serum levels of ferritin, and serum levels of iron using automated hematology, immunology, and chemistry analyzer. ERFE and hepcidin were measured by a specific enzyme-linked immunosorbent assay kit. RESULTS: The serum ERFE levels were higher than normal in all cases and were negatively correlated with serum hepcidin (r = −0.023). In IDA, serum ERFE concentration had a nonsignificant negative correlation with hemoglobin (Hb) concentration. Serum hepcidin concentration had a nonsignificant negative correlation with Hb concentration. Serum ERFE had a nonsignificant negative correlation with Hb% in severe IDA (r = −0.679; P = 0.094) and mild IDA (r = −0.068; P = 0.789). ERFE had a nonsignificant positive correlation with Hb% in moderate IDA (r = 0.069; P = 0.793). Serum hepcidin had a nonsignificant positive correlation with Hb% in severe IDA (r = 0.036; P = 0.939). Serum hepcidin had a nonsignificant negative correlation with Hb% in mild IDA (r = −0.079; P = 0.764) and moderate IDA (r = −0.179; P = 0.491). CONCLUSIONS: The potential of ERFE and hepcidin in diagnosing and categorizing ID disorders is promising. Understanding the mechanism of ERFE/hepcidin interaction will help in developing ERFE-/hepcidin-targeted therapies.

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BACKGROUND: Over the years, with changes in treatment approaches, it has been possible to achieve higher complete response (CR) rates with chemotherapies or chemoimmunotherapies in multiple myeloma (MM). However, a subset of patients in CR still relapse owing to the presence of residual tumor cells in the bone marrow not detectable by conventional methods. Residual disease detection by flow cytometry (FCM) has been proven to be highly sensitive and prognostically significant in a number of clinical studies. AIMS AND OBJECTIVES: In this study, we compared FCM minimal residual disease (FCM MRD) in MM cases post-chemotherapy/autologous stem cell transplant with morphology and biochemical methods. We also tried to correlate the pre-therapy stage of the disease and cytogenetics with MRD. MATERIALS AND METHODS: Twenty eight samples from 26 patients were evaluated for MRD on 6 color 3 tube panel over the period of 2 years. RESULTS: MRD was detectable in 19 samples (67.9%). FCM had a sensitivity of 95% compared to immunohistochemistry (IHC). 100% of cases with MRD positivity had abnormalities in at least three surface antigens. The high risk cytogenetics and high risk stage groups had a higher frequency of MRD positivity compared to the low risk groups. CONCLUSION: FCM MRD analysis is able to risk stratify the patients in CR and stringent CR. Routine use of FCM to detect residual disease posttherapy in MM should be implemented.

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BACKGROUND: Cancer is considered a major risk factor of venous thromboembolism (VTE). Whether the D-dimer level at first incident cancer-associated VTE can predict recurrence is not fully elucidated. AIM: The aim of this study was to assess the association between D-dimer level measured at first incident cancer-associated VTE and risk of recurrence. METHODS: In this study, the electronic records of all patients with first incident cancer-associated VTE were retrospectively retrieved and followed up for a period of 36 months. The measured levels of D-dimer and clinical predictors for each patient prior intervention were collected. Univariate and multivariant Cox regressions were fitted to estimate the hazard ratio (HR) and 95% confidence interval (CI). RESULTS: A total of 73 (34.3%) of 213 cancer-associated VTE patients had recurrent VTE. The crude recurrence rate was 11.4/100 person-years (95% CI, 9.2–14.2). The mean value of D-dimer was significantly higher seen in recurrent VTE patients than those without recurrence (14.8 ± 7.5 mg/L vs. 4.8 ± 5.7 mg/L, P < 0.001). The highest positive likelihood ratio using area under the receiver operating characteristic (ROC) curve occurred when a D-dimer threshold of 8.67 mg/L was chosen. At this threshold, the D-dimer was 92% sensitive and 80% specific in predicting recurrent VTE. The area under the ROC curve was 0.924 (95% CI 0.887–0.960, P < 0.001). Patients with a D-dimer ≥8.67 mg/L at first VTE in our study were 3.1-fold more likely to have a recurrence than those with lower D-dimer results. CONCLUSION: A D-dimer ≥8.67 mg/L measured at the first incident cancer-associated VTE was associated with a 3.1-fold increased hazard of recurrence events. Our finding suggests that a low D-dimer level at the time of first cancer-associated VTE is suitable in the clinical practice to avoid extended-duration anticoagulation.

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BACKGROUND: COVID-19 as a pandemic has caused an alarming increase in mortality and morbidity. Viral-induced morphologic changes in the peripheral blood cells are well characterized in certain infections and can direct diagnostic workup to ensure timely therapeutic intervention. This study describes the morphological changes of blood cells in various stages of COVID disease. MATERIALS AND METHODS: A total of thousand COVID-positive patients admitted in the tertiary care center were taken for the study. They were classified as mild, moderate, and severe based on the clinical criteria suggested by World Health Organization. Peripheral smears of the patients were analyzed, and the morphological changes in various blood cells were correlated with the disease stage and coagulation parameters. RESULTS: The study demonstrated significant morphological changes in the blood cells of COVID patients during the course of disease progression and during the onset of COVID-associated coagulopathy. Leukocytosis, neutrophilia, and toxic changes in neutrophils were seen in the severe stage of the disease and in COVID coagulopathy suggesting these are important indicators of disease severity. Activated lymphocyte was found to be the most common morphological presentation seen in all patients irrespective of the disease stage, whereas plasmacytoid lymphocytes were an important finding in severe-stage disease. Schistocytes an important finding in any other coagulopathy was present only in 1% of cases of COVID coagulopathy. CONCLUSIONS: The study demonstrated significant morphological changes in the blood cells of COVID-positive patients during the course of disease progression. Comprehensive daily complete blood count and peripheral smear examination should be undertaken in patients hospitalized with COVID-19 to predict potential clinical deterioration and signs of disease progression. These morphological changes in peripheral smear can be used as one of the factors indicating disease progression which can formulate for further evaluation. Since follow-up and post-COVID morphological examination were not done, additional research in this aspect can shed light on the clinical categorization of COVID patients based on the morphological findings.

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The current coronavirus disease 2019 (COVID 19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS CoV 2), has impacted many facets of hematopoietic cell transplantation (HCT) in both developed and developing countries. The full impact of the COVID 19 pandemic, caused by the SARS CoV 2, on the field of hematopoietic cell transplantation (HCT) is unknown. Here, we report a rare case of a 21-year-old male patient known to have chronic myeloid leukemia (CML) with progression to T-cell lymphoblastic lymphoma as extramedullary blast crisis of CML. The patient was treated by pediatric chemotherapy regimen then. He underwent haploidentical stem cell transplantation. Posttransplantation, on day +7, he developed SARS-CoV-2 after receiving stem cell graft from a donor who was diagnosed with SARS-CoV-2 on the day of stem cells harvesting. The case elaborates complications and outcome of a patient receiving stem cell transplant from a donor with SARS-CoV-2 infection.

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The loss of CD38 expression has been described in multiple myeloma (MM) patients with relapsed/refractory disease and patients receiving CD38 monoclonal antibodies. However, the loss of CD38 at diagnosis is extremely rare with very few reports describing such finding in newly diagnosed MM patients. Only two detailed case reports described CD38-negative clonal plasma cells at diagnosis. However, none of these cases was primary plasma cell leukemia (PCL). We describe an elderly patient with primary PCL presenting mainly with a negative CD38 expression, which, to the best of our knowledge, has never been described. The patient did not do well and died within 3 months of diagnosis. The loss of CD38 expression at diagnosis could indicate more aggressive disease biology.

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We report a rare case of a 58-year-old woman who was initially diagnosed as primary gastric (PG) T-cell non-Hodgkin lymphoma, which later was revised as PG Hodgkin lymphoma (HL) with dissemination to the lung (stage IV). The lung lesion was later found to be lung adenocarcinoma (LA) and the diagnosis was revised again as synchronous primary gastric Hodgkin lymphoma (PGHL) and LA which, to our knowledge, was not reported before. This case illustrated the need of vigilance in the diagnosis of lymphoma at uncommon sites and the clinical challenges in managing synchronous malignancies. The rationale of clinical suspicion, the association between PGHL and Epstein–Barr virus, Helicobacter pylori, and other infections, and synchronous PGHL and LA were discussed.

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Hemophagocytic lymphohistiocytosis (HLH) is a rare and complex disorder that involves numerous hematological and immunological reactions. These reactions, if left untreated, may lead to multiorgan failure and death. Corticosteroids are the mainstay of HLH therapy which may also comprise other chemotherapeutic or immunotherapy agents. The components of the 2004 HLH diagnostic criteria may intersect with several other conditions. In this report, we present the case of a 4-year-old boy who was diagnosed with Epstein–Barr virus-related infectious mononucleosis complicated by clinical and laboratory features consistent with HLH, which self-resolved completely without HLH-directed therapy.

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